Girl with two hearts healthy after loss of one

A British girl has recovered fully after spending 10 years with an extra heart transplanted alongside her own.

Surgeons this week described in detail how they saved her life twice, first by transplanting the heart, and then by taking it out again 10 years later.

Now 16 years old, Hannah Clark received a second heart in 1995 at the age of two to do the work of her own heart – the muscles of which were rapidly failing through cardiomyopathic disease.

Ten years after the operation, her own heart appeared to have fully recovered, but the donor heart – originally taken from a 5-month-old baby girl – was flagging.

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Moreover, Hannah’s life was again threatened, this time by a type of lymph cancer caused by an otherwise dormant virus, called the Epstein-Barr virus. The cancer struck four times altogether, mainly because immunosuppressive drugs to stop the donated heart being rejected prevented Hannah’s immune system from keeping the virus in check.

So in February 2006, the second heart was removed, Hannah’s immunosuppressive drugs halted, and within days her cancer disappeared, apparently for good.

‘Magical recovery’

On Monday, she appeared with her family at a press conference in London alongside the two surgeons who had saved her life.

“The possibility of recovery of the heart is like magic,” says Magdi Yacoub, the heart surgeon at Harefield Hospital in Middlesex, UK who performed the operations. “Now, her heart is functioning normally.”

Tsang says that about 50 patients had received second hearts between 1974 and 1983 in a “piggyback” procedure first developed by Christiaan Barnard, the South African heart surgeon who performed the world’s first heart transplant in 1967.

Backup plan

The principle behind using a pair of hearts was that if the transplanted heart failed through rejection, the patient’s own could sustain life till another transplant was available. But the procedure was superseded once powerful immunosuppressive drugs were available, and has largely been abandoned since the mid 1980s.

Hannah, though, was an exception because of her circumstances. Giving her a second heart when she was two was the only way to save her life. No heart of the right size was available, ruling out a straight swap, and resistance to blood flow from her lungs was causing her to deteriorate, so time was running out.

So when a much smaller heart became available – from a baby half Hannah’s weight at the time – her medical team decided the only life-saving option was to plumb the heart directly into Hannah’s to make a double heart.

For the first few months, the new heart did nearly all the work, but this “rest” gave Hannah’s own heart a chance to begin recovery. By the time of the second heart was finally removed, Hannah’s heart was doing virtually all the work itself.

Wasted hearts

But now the viral cancer, called post-transplant lymphoproliferative disorder (EBV-PTLD), was threatening Hannah’s life, with cancerous swellings in her windpipe obstructing her breathing to the point where she needed to be put on a ventilator.

So Yacoub and Tsang had to weigh up the balance between retaining the second heart to continue helping Hannah’s own heart and taking it out so that the immunosuppressive drug regime could be halted and the cancer destroyed.

In the end, they opted to take out the heart, thankfully leading to a full recovery.

The surgeons say that mechanical hearts are now more widely available to act as “auxiliary pumps” in patients with cardiomyopathies until a suitable donor comes along. However, a quarter die still while waiting. This toll could be cut, they say, if smaller hearts were used as in Hannah’s case, to do some but not all of the work. At present, many such hearts are discarded because they are the wrong size.